Frequency, treatment, and functional outcome in children with hypersensitivity pneumonitis

Background Hypersensitivity pneumonitis is a rare interstitial lung disease and very few data regarding frequency, treatment and outcome exist for children. Children identified with hypersensitivity pneumonia from a Danish national cohort with diffuse interstitial lung disease form the basis of this...

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Published in:Pediatric pulmonology 2011-11, Vol.46 (11), p.1098-1107
Main Authors: Buchvald, Frederik, Petersen, Bodil Laub, Damgaard, Karen, Deterding, Robin, Langston, Claire, Fan, Leland L., Deutsch, Gail H., Dishop, Megan K., Kristensen, Lene Agergaard, Nielsen, Kim G.
Format: Article
Language:English
Subjects:
Adolescent
Alveolitis, Extrinsic Allergic - drug therapy
Alveolitis, Extrinsic Allergic - epidemiology
Alveolitis, Extrinsic Allergic - pathology
Anti-Inflammatory Agents - therapeutic use
Biological and medical sciences
Child
Child, Preschool
children
Chronic Disease
Denmark - epidemiology
Female
General aspects
Humans
hypersensitivity pneumonitis
Incidence
Infant
Male
Medical sciences
Methylprednisolone - therapeutic use
outcome
Pneumology
Prevalence
Respiratory Function Tests
Respiratory system : syndromes and miscellaneous diseases
Retrospective Studies
treatment
Treatment Outcome
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Summary:Background Hypersensitivity pneumonitis is a rare interstitial lung disease and very few data regarding frequency, treatment and outcome exist for children. Children identified with hypersensitivity pneumonia from a Danish national cohort with diffuse interstitial lung disease form the basis of this study focused on disease frequency, treatment, and functional outcome. Methods Seventy‐three children with clinical and radiological signs of interstitial lung disease verified by lung biopsy were identified over a 12‐year period. Histologic material from all cases was reviewed by pathologists from the ChILD Clinical and Research Network, USA. Diagnosis of hypersensitivity pneumonitis was confirmed in 19 cases. Measurements and Main Results Incidence of hypersensitivity pneumonitis was approximately 2/year and with a point prevalence of 4/1,000,000 children. The median (range) number of monthly courses with intravenous methylprednisolone was 15 courses (8–34) in resolved cases, but in the vast majority (92%), mono‐therapy with high dose pulse methylprednisolone treatment was not sufficient for acceptable improvement. Lung function, DLco and DLco/VA increased significantly after 3 and 6 months of treatment compared to baseline (P 
ISSN:8755-6863
1099-0496
DOI:10.1002/ppul.21479